Conditions · Gastroenterology and hepatology

Budd-Chiari syndrome !

A blockage of the veins that drain blood out of the liver. Blood backs up, causing a painful enlarged liver, ascites and possible liver failure.

Updated 2026-11-01

Structures involved

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In the clinical exam

Why it happens

What it is

Obstruction of hepatic venous outflow at any level from the small hepatic veins to the junction of the inferior vena cava with the right atrium, most often by thrombosis. Congestion of the liver causes hepatomegaly, ascites and liver damage.

Who gets it

Rare; most common in young adults, more often women. In Western countries thrombosis linked to myeloproliferative neoplasms predominates, whereas in Asia membranous obstruction of the vena cava is more frequent. Cases in Latin America mirror the thrombotic pattern.

Causes

Primary: thrombosis of the hepatic veins or suprahepatic inferior vena cava
Secondary: compression or invasion by tumours (hepatocellular, renal cell carcinoma), abscesses or cysts
Membranous webs of the inferior vena cava

Risk factors

Myeloproliferative neoplasms, especially polycythaemia vera (JAK2 mutation)
Inherited thrombophilia (factor V Leiden, protein C or S deficiency) and antiphospholipid syndrome
Paroxysmal nocturnal haemoglobinuria
Oral contraceptives, pregnancy and the postpartum period
Behçet disease and other inflammatory conditions

Pathophysiology

Blocked outflow raises sinusoidal pressure, causing congestion, hepatomegaly and ascites with a high protein content. Centrilobular hypoxia leads to necrosis and, over time, fibrosis ('nutmeg liver'). The caudate lobe drains directly into the vena cava, so it is spared and enlarges.

Classification

Fulminant (acute liver failure)
Acute (pain, ascites, hepatomegaly over weeks)
Subacute or chronic (most common; insidious ascites, may present as cirrhosis)
By level: hepatic veins, inferior vena cava, or both

Symptoms and signs

Classic triad: right upper quadrant abdominal pain, hepatomegaly and ascites
Jaundice in acute forms
Leg oedema and dilated veins on the trunk and back when the inferior vena cava is blocked
Splenomegaly and other signs of portal hypertension
May be asymptomatic when collaterals have developed

Diagnosis

Clinical: Suspected in a patient with painful hepatomegaly and ascites, particularly with a thrombotic disorder, or in unexplained liver disease or acute liver failure.
Tests: Liver tests and INR; ascitic fluid with a high protein level and a serum-ascites albumin gradient ≥1.1 g/dL. Work-up for the cause: JAK2 V617F mutation, blood count, thrombophilia screen, antiphospholipid antibodies and PNH flow cytometry.
Imaging: Doppler ultrasound is the first-line test: absent or reversed flow in hepatic veins, thrombus, intrahepatic collaterals and a hypertrophied caudate lobe. CT or MRI confirms and maps the obstruction; venography guides intervention.

Differential diagnosis

Cirrhosis from other causes
Right heart failure or constrictive pericarditis (congestive hepatopathy)
Sinusoidal obstruction syndrome (after chemotherapy or stem-cell transplant)
Portal vein thrombosis
Peritoneal tuberculosis or malignancy as causes of ascites

Goal of treatment

Stop clot extension with anticoagulation, treat the underlying cause, control ascites and restore outflow with a step-up approach (angioplasty, TIPS, transplant) when the liver does not improve.

Treatment

Medical: Lifelong anticoagulation (heparin, then oral anticoagulants) for all patients unless contraindicated; diuretics and sodium restriction for ascites; treatment of the underlying disorder, such as cytoreduction for a myeloproliferative neoplasm.
Procedure: Angioplasty with or without stenting for short venous stenoses or caval webs; local thrombolysis for recent clots in selected cases.
Procedure: TIPS (transjugular intrahepatic portosystemic shunt) when anticoagulation and angioplasty fail.
Surgical: Liver transplantation for fulminant presentation or failure of TIPS.
Follow-up: Anticoagulation levels and bleeding signs, abdominal girth and weight, liver tests, and surveillance for hepatocellular carcinoma in chronic disease.

Complications

Acute liver failure
Portal hypertension with variceal bleeding
Cirrhosis
Hepatocellular carcinoma
Bleeding from anticoagulation

Prognosis

With anticoagulation and the step-up strategy, most patients now survive long term; five-year survival is reported around 80–90%. Fulminant cases and those with severe underlying disease do worse.

Test yourself

Think of the answer, then tap the question to see it.

What is the classic triad of Budd-Chiari syndrome?

Right upper quadrant abdominal pain, hepatomegaly and ascites.

Which underlying disorder is the most common cause in Western series?

A myeloproliferative neoplasm, especially polycythaemia vera with the JAK2 V617F mutation.

Why does the caudate lobe enlarge in Budd-Chiari syndrome?

It drains directly into the inferior vena cava through its own veins, so it is spared and hypertrophies.

What is the first-line imaging test?

Doppler ultrasound of the hepatic veins and inferior vena cava.

What is the stepwise treatment of Budd-Chiari syndrome?

Anticoagulation, then angioplasty or stenting, then TIPS, and finally liver transplantation.

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Educational information, not medical advice. For any symptom, see a health professional.

Sources: Hepatic vein obstruction (Budd-Chiari): MedlinePlus Medical Encyclopedia (MedlinePlus (U.S. National Library of Medicine), 2024).

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Representative model, for educational purposes only.

Hepatic veins highlighted on the Durgical 3D model